Pretreatment and posttreatment positron emission tomographic scan imaging in a 20-year-old patient with Wilson's disease.
Wilson's disease is an autosomal-recessive inherited disorder that results in predominantly hepatic and neurologic manifestations. Neurologic abnormalities include tremor, ataxia, bradykinesia, rigidity, chorea, and dystonia. We report the clinical, radiologic, and serial FDG PET findings in a 20-year-old woman who presented with an asymmetric upper limb tremor caused by Wilson's disease. Reduced striatal and cerebral cortical glucose metabolism was demonstrated on a FDG PET study performed before the commencement of D-penicillamine therapy. After 6 months of treatment, the patient had shown only minimal clinical improvement, despite an increase in striatal and cerebral cortical glucose metabolism on a repeat FDG PET study. After 14 months of treatment, however, a moderate clinical improvement was noted and there was further increase in glucose metabolism on FDG PET.
Cordato
D J
DJ
Department of Neurology, Concord Hospital, Sydney, Australia.
Fulham
M J
MJ
Yiannikas
C
C
D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D002540:Cerebral Cortex / Q000000981:diagnostic imaging / Q000187:drug effects / Q000378:metabolism*; D002614:Chelating Agents / Q000627:therapeutic use*; D003342:Corpus Striatum / Q000000981:diagnostic imaging / Q000187:drug effects / Q000378:metabolism*; D005260:Female; D005947:Glucose / Q000378:metabolism; D006527:Hepatolenticular Degeneration / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000378:metabolism*; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D010396:Penicillamine / Q000627:therapeutic use*; D014055:Tomography, Emission-Computed
D002614:Chelating Agents; D010396:Penicillamine; D005947:Glucose